5612992 SICKLE CELL HEALTH AWARENESS, PERSPECTIVES, AND EXPERIENCES (SHAPE) SURVEY: FINDINGS ON THE BURDEN OF SICKLE CELL DISEASE AND IMPACT ON THE QUALITY OF LIFE OF PATIENTS AND CAREGIVERS IN THE UK
Authors: B.P.D. Inusa, J. James, B. Tinga, D. Ba, E. Ingoli, R. Hartfield, A. Anderson, F.F. Costa, W. Jastaniah, J.B. Kunz, I. Odame, A. Beaubrun, B. Lartey, M. de Montalembert
Journal: HemaSphere (2023)

Background: Sickle cell disease (SCD) substantially impacts the physical and emotional well-being of patients and their caregivers, yet research of the impact of SCD on quality of life (QOL) is limited. Approximately 15,000 people in the United Kingdom (UK) live with SCD, and 1 in 2517 babies in England were diagnosed with SCD between 2019 and 2020, warranting the need to identify and address the challenges faced by individuals with SCD and their caregivers in this region of the world. The SHAPE survey aims to improve our understanding of the global impact of SCD on patients and their caregivers. This analysis reports survey findings from the UK within the context of insights obtained internationally. Aims: To understand the impact of SCD on the QOL of patients and caregivers in the UK. Methods: SHAPE was an online survey administered to patients and caregivers from the UK, Germany, France, US, Brazil, Canada, Saudi Arabia, United Arab Emirates, Bahrain, and Oman (patients only). Patients with SCD aged ≥12 years and caregivers aged ≥18 years supporting a patient with SCD were eligible. Participants completed a 12-minute online survey comprising a range of close-ended questions regarding their circumstances and experiences to build a robust and reliable dataset on which descriptive statistics were performed. Results: Of 919 patients and 207 caregivers interviewed globally, 151 patients and 30 caregivers were from the UK. Fatigue/tiredness (84%) and vaso-occlusive crises (VOCs; 71%) were the symptoms most frequently experienced by patients from all 10 countries. In the UK, a comparable percentage of patients experienced fatigue/tiredness (90%), while a higher percentage experienced VOCs (81%). In addition, the symptoms more frequently reported by patients in the UK (≥60%) than all patients were low mood/feeling down/depressed (74% UK vs 62% total), poor sleep/insomnia (72% UK vs 60% total), headache (71% UK vs 60% total), poor mobility/movement (70% UK vs 46% total), and joint stiffness (62% UK vs 50% total). Patients in the UK missed a similar number of days of school or work in 1 month to all patients (mean, 7.9 UK vs 7.5 total). Compared with all patients, a greater percentage of patients in the UK were worried about being seen less competent than others at school/work (72% vs 63% total). The symptoms experienced by all patients with SCD that most impacted caregivers’ lives were fatigue/tiredness (49%) and VOCs (43%), while caregivers in the UK reported a lower impact of fatigue/tiredness (27%) and a greater impact of VOCs (77%). More than half of the caregivers in the UK felt that caring for someone with SCD affected their earning potential (57% vs 54% total) and the ability to attend and succeed at school or work (53% vs 56% total). Caregivers in the UK missed an average of 6.1 days of school or work in a month vs 5.0 days among all caregivers. Summary/Conclusion: The findings reported in this survey highlight the symptoms of SCD that are perhaps a greater concern for patients in the UK compared with patients globally, emphasizing the need for symptom management to improve the QoL of affected individuals in the UK. Compared with all caregiver respondents, more caregivers in the UK identified VOCs as having the greatest impact on their lives, limiting the earning potential and ability to attend school or work. Overall, these insights highlight areas that require improved patient and caregiver support. Reference 1. Osunkwo et al, Am J Hematol 2021; 96: 404